Pulmonary Fibrosis

In the Cambridge Dictionary, the definition of fibrosis is as follows: “the formation of too much fibrous tissue as a result of healing, inflammation or irritation.”

Interstitial Lung Disease (ILD) is a blanket term to describe many diseases that cause fibrosis (scarring) of the lungs. The scarring is pulmonary fibrosis.

More than two hundred types of Interstitial Lung Disease (ILD) exist.

The interstitium is a layer of lung tissue in the alveoli walls (clusters of air sacs within the lung). Fibrosis scars and thickens the interstitium. As a result, the air sacs are less flexible, and the lung becomes stiffer, making the air flow in and out of the lungs more difficult.

People with pulmonary fibrosis become short of breath, leading to extreme tiredness, even after rest. Typically, but not always, this is accompanied by a dry cough.

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What are the causes of pulmonary fibrosis?

• Occupational exposure to toxic dust

Known causes of pulmonary fibrosis include exposure to certain types of dust and pollutants in an occupational setting, such as:

• Coal dust.
• Silica.
• Asbestos fibres.
• Grain dust.

Coal dust, when inhaled, causes coal workers’ pneumoconiosis. Silica dust inhalation can lead to silicosis. Asbestos exposure causes interstitial fibrosis or, as it is more commonly known, asbestosis. Asbestosis is often incorrectly used as an umbrella term for all types of asbestos-related diseases. It is an asbestos disease in its own right.

• Collagen vascular diseases

Pulmonary fibrosis is often diagnosed in people with collagen vascular diseases such as:

• Scleroderma.
• Lupus.
• Rheumatoid arthritis.
• Polymyositis.
• Sjogren’s syndrome.
• Sarcoidosis.

• Idiopathic Pulmonary Fibrosis

Two-thirds of all pulmonary fibrosis cases cannot identify a known cause, and these are known as Idiopathic Pulmonary Fibrosis (IPF).

It is thought that hereditary factors may play a part in developing IPF, but there are numerous other theories about what could cause this form of pulmonary fibrosis. Some possible triggers include:

• Viruses.
• Tobacco smoke.
• Some types of medication.
• As a side effect of radiation treatment.

Usual interstitial pneumonitis (UIP) and cryptogenic fibrosing alveolitis (CFA) are other types of fibrosis with no known cause.

It is estimated that around 30,000 people in the UK have IPF, and over 5000 people die yearly from the disease.

What are the symptoms of pulmonary fibrosis?

Pulmonary fibrosis causes stiffening of the lungs, thus making it difficult for someone with the disease to breathe in and out. Breathlessness, accompanied by a lack of energy and extreme fatigue, is the most worrying and debilitating of symptoms for the sufferer. A dry crackling cough is one of the earliest symptoms as pulmonary fibrosis takes hold.

Weight loss with no other recognised cause and aching joints and muscles often accompany pulmonary fibrosis.

The disease is progressive, meaning that it will get worse over time. Healthcare professionals advise that attempting to maintain as healthy a lifestyle as possible is one of the most effective means of slowing down the progression of the disease.

How is pulmonary fibrosis diagnosed?

It is likely to be the combination of a dry cough and extreme breathlessness that instigates a visit to their GP for the possible PF sufferer. The crackling in the chest will be recognisable upon investigation by the GP as a possible sign of pulmonary fibrosis.

Referral for a chest x-ray is the next likely step in the diagnosis process. If a lung disorder is suspected, a CT scan, a lung function test, and a biopsy will all occur if necessary.

What treatment can be given to pulmonary fibrosis patients?

Treatment options for PF patients are limited. There is no cure for pulmonary fibrosis and no means of reversing the scarring that has already occurred.

Available treatments focus on reducing the rate at which other scar tissue forms so that symptoms can be managed.

Pulmonary fibrosis patients are often provided with oxygen tanks at home so that they can self-administer additional oxygen utilising breathing apparatus attached to the tanks. This can go some way to help alleviate breathlessness.

To what extent symptoms progress varies from individual to individual. For some, treatment will slow the progression of the disease markedly, and for others, symptoms will rapidly get worse.

What is the prognosis for pulmonary fibrosis?

As mentioned above, the scarring of the lungs that has already taken place cannot be reversed – there is no cure for pulmonary fibrosis.

If you have worked in an occupation where you regularly came into contact with asbestos, then be sure to visit your GP if you begin to experience what may be pulmonary fibrosis symptoms.

In particular, should you develop increased breathlessness that cannot be explained away as something else, you must see your GP for a check-up. When you speak with health professionals about the symptoms you are experiencing, it is essential to tell them about your history of working with asbestos.

By making the health professionals aware that you suffered asbestos exposure, you can help to ensure your condition is not wrongly diagnosed as IPF when, in fact, you have the early onset of asbestosis.

Pulmonary Fibrosis Claims Solicitor UK

Being faced with a pulmonary fibrosis diagnosisis is an overhwhelming experience and one that can feel highly daunting. When faced with such reality, it is essential to seek the appropriate legal advice and support in order to achieve the compensation that you deserve.

Bridge McFarland LLP have a team of dedicated personal injury solicitors with specialist knowledge in asbestos-related disease claims.

Asbestos Awareness is dedicated to informing, educating, and supporting people in relation to asbestos-related diseases.

For further information, or to speak to a member of our experienced medical personal injury solicitors, please call 0800 987 8809. Alternatively, you can fill in this contact form.

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